The Great Mimicker: Langerhans Cell Histiocytosis Mimicking Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy.

IF 0.7 4区 医学 Q4 PATHOLOGY
Tanvi Jha, Prajwala Gupta, Varun Garg, Vivek Dewan
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引用次数: 0

Abstract

Langerhans Cell Histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, often affecting multiple organ systems and mimicking conditions like autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED). This case report details a 3-year-old male, who presented with submandibular swellings, nail abnormalities, seborrheic dermatitis and systemic involvement, initially diagnosed as APECED syndrome clinically. Fine-needle aspiration cytology (FNAC) and immunohistochemistry confirmed LCH. The patient was treated with vinblastine and prednisolone and showed marked improvement. This case emphasizes the importance of early, accurate diagnosis on FNAC to differentiate LCH from other mimicking conditions, enabling timely treatment and improved prognosis.

伟大的模仿者:朗格汉斯细胞组织细胞增多症模拟自身免疫性多内分泌病念珠菌病外胚层营养不良。
朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的朗格汉斯细胞增生性疾病,通常影响多器官系统,并模仿自身免疫性多内分泌病念珠菌病外胚层营养不良(APECED)等疾病。本病例报告详细介绍了一名3岁男性,他表现为下颌肿胀,指甲异常,脂溢性皮炎和全身受累,最初临床诊断为APECED综合征。细针穿刺细胞学(FNAC)和免疫组织化学证实LCH。患者经长春碱联合强的松龙治疗,病情明显好转。本病例强调了FNAC早期准确诊断的重要性,以区分LCH与其他类似疾病,及时治疗和改善预后。
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来源期刊
CiteScore
3.00
自引率
0.00%
发文量
68
审稿时长
6-12 weeks
期刊介绍: Fetal and Pediatric Pathology is an established bimonthly international journal that publishes data on diseases of the developing embryo, newborns, children, and adolescents. The journal publishes original and review articles and reportable case reports. The expanded scope of the journal encompasses molecular basis of genetic disorders; molecular basis of diseases that lead to implantation failures; molecular basis of abnormal placentation; placentology and molecular basis of habitual abortion; intrauterine development and molecular basis of embryonic death; pathogenisis and etiologic factors involved in sudden infant death syndrome; the underlying molecular basis, and pathogenesis of diseases that lead to morbidity and mortality in newborns; prenatal, perinatal, and pediatric diseases and molecular basis of diseases of childhood including solid tumors and tumors of the hematopoietic system; and experimental and molecular pathology.
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